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Multiple and huge hemangiomas - Practical hematology of children's age

Table of contents
Practical hematology of children's age
Embryonal hemopoiesis
Morfofunktsionalny characteristic of cells of marrow and peripheral blood
Marrow parenchyma cells
Peripheral blood of children of different age
The system of a hemostasis is normal
Etiology and pathogeny of leukoses
Acute leukoses
Acute leukoses - a preleukosis
Possibilities of a predictive assessment of a course of an acute lymphoblastoid leukosis at children
General principles of treatment of an acute leukosis
Chemotherapeutic drugs
Treatment of an acute lymphoblastoid leukosis
Treatment of myeloid forms of an acute leukosis
Infectious complications and symptomatic therapy of an acute leukosis
Consolidation and maintenance therapy of an acute leukosis
Immunotherapy
Remission and recurrence of an acute leukosis
Inborn leukosis
Neuroleukosis
Myelosis
Lymphogranulomatosis
Gematosarkoma
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Leukopenias
Histiocytoses
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Angiopathies
Hemorrhagic vasculitis (Shenleyn's illness — Genokh)
Mayokki's purpura
Ataxy teleangiectasia
Entsefalotrigeminalny angiomatosis
Kortiko-meningealny diffusion angiomatosis
Cerebroretinal angiomatosis
Hypertrophic gemangiektaziya
Multiple and huge hemangiomas
Elastic fibrodisplaziya
Coagulopathies
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Angiohemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Dysfibrinogenemias
Hereditary deficit of factors of VII, X, V and II
Deficit K-vitaminozavisimykh of factors of coagulation
Syndrome of the disseminated intravascular coagulation
Clinic and diagnosis of the IDCS
Treatment of the IDCS
Thrombocytopenia
Idiopathic Werlhof's disease
Clinic and diagnosis of an idiopathic Werlhof's disease
Treatment of an idiopathic Werlhof's disease
Isoimmune Werlhof's disease
Transimmune Werlhof's disease of newborns
Trombogemolitichesky Werlhof's disease (syndrome Moshkovich)
Hereditary Werlhof's diseases
Trobotsitopatiya
Anemias
The anemias connected with blood loss
Chronic posthemorrhagic anemia
Iron deficiency anemias
Clinic and diagnosis of an iron deficiency anemia
Treatment of iron deficiency anemias
Sideroakhrestichesky, sideroblastny anemias
Megaloblastny anemias
Foliyevodefitsitny anemia
Hereditary forms of megaloblastny anemias
Hereditary dizeritropoetichesky anemias
The anemias connected with oppression of proliferation of cells of marrow
Hereditary hypoplastic anemias
Hemolitic anemias
Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis
Acanthocytosis, piknotsitoz
The hereditary hemolitic anemias connected with disturbance of activity of enzymes of erythrocytes
The hereditary hemolitic anemias connected with disturbance of structure or synthesis of hemoglobin
The acquired immune hemolitic anemias
Isoimmune hemolitic anemias
Treatment of a hemolitic illness of newborns
Autoimmune hemolitic anemias
List of references

Multiple hemangiomas

B1960 of of I. D. Rily and A. Smith described a syndrome at which the combination of skin hemangiomas to a macrocephalia, Riley who received the name of an illness — Smitna is observed. The syndrome is caused by a dysplasia of vascular system and mesenchymal fabric. It is inherited on autosomal dominant type. From the moment of the birth at the sick child the head increases in sizes, however without symptoms of hydrocephaly or increase of intracranial pressure. Also there is no neurologic symptomatology. Within a year on skin there are hemangiomas which tend to growth. Forecast of a disease rather favorable. An effective method of treatment is radical removal of hemangiomas.
In 1963. T. Gluszcz and coauthors described a syndrome at which there is a generalized dysplasia of connecting fabric and vascular system. Assume an autosomal and recessive mode of inheritance. The disease arises at early age. Skin hemangiomas, looseness of joints, disturbance of structure of bodies of vertebrae are noted. The forecast is rather favorable.

The huge hemangiomas which are followed by thrombocytopenia (Kazabakh's syndrome — - Merritt)

The disease is in detail described in 1940 by N. N. Kasabach and K. K. Merritt. Hereditary character it is not established. With an identical frequency persons man's and female are ill, arises at early age. At pathoanatomical research find the vascular tumor having a cavernous structure and consisting of a set of the cavities filled with blood. Bleeding is caused by existence of a hemangioma. Studying of a hemostasis revealed the constant thrombocytopenia and a fibrinogenopenia reaching sometimes afibrinogenemiya degree. The processes happening in a hemangioma on the mechanism are a chronic form of the IDCS. In the huge hemangioma having an extensive plentiful vascular network there is a delay of a blood-groove to formation of multiple microblood clots and the raised expenditure of thrombocytes. As a result of it the secondary thrombocytopenia and a consumption coagulopathy connected with increase of a blood coagulation in a hemangioma develop. Further developing of thrombocytopenia is promoted by decrease in marrowy products as there is functional exhaustion of reserves connected with the continuous increased destruction of thrombocytes in a hemangioma.
Hemangiomas find in children at early chest age. Preferential localization — on extremities, a trunk, is more rare on a face; they are distinguished by rapid growth. Hemangiomas can be located in bones of a skeleton, a liver, a brain. Except a huge hemangioma, some patients have small angiomas without tendency to growth. As a rule, bleedings arise spontaneously.
Along with bleedings there are other hemorrhagic manifestations characteristic of a Werlhof's disease. In a circle of a hemangioma and on all body petechias and extensive extravasates appear, they can be also marked out on mucous membranes. At heavy thrombocytopenia there can be bleedings from mucous membranes of a nasal cavity, a digestive tract. The forecast considerably becomes complicated at hemorrhages in vitals. At a blood analysis reveal thrombocytopenia, posthemorrhagic anemia, anemia can sometimes have hemolitic character. The bleeding time increases, retraction of a blood clot is broken, the fibrinogenopenia is found.
The forecast of a disease is rather serious. Therapy of a syndrome of Kazabakh — Merritt is carried out in two directions. The radical oncotomy contributes to normalization of quantity of thrombocytes and disappearance of a hemorrhagic syndrome. The roentgenotherapy leading to reduction of a hemangioma and reduction of hemorrhagic manifestations gives a positive effect. Conservative therapy of the acute period is directed to a stop of bleeding and maintenance of life activity of an organism. Carry out transfusions of blood, a platelet concentrate, apply symptomatic means. Therapy by corticosteroid hormones as a result of which it is possible to receive kliniko-hematologic remission gives a certain effect. Hormonal therapy is also shown by preoperative preparation.



 
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